Immunohistochemical study was performed using monoclonal antibodies specific to neurofilament (NF) (clone NR4 and 2F11) and synaptophysin (SY) in the bowel of Hirschsprung's disease and related diseases. The following results were obtained. 1) The ganglionic bowel : NF-immunoreactivities by NR4 were localized in the plexuses but weak in distal axons, but those by 2F11 were localized in the plexuses and distal axons. SY-immunoreactivities were localized in the plexuses and muscle layers. 2) The aganglionic bowel : NF-immunoreactivities by NR4 and 2F11 were localized in hypertrophic axon bundles. NF-immunoreactive nerve fibers decreased from the rectum to the descending colon in long segment aganglionosis. SY-immunoreactivities were localized in muscle layers but decreased gradually in more proximal areas. 3) The bowel of hypoganglionosis : In the case 1, NF-immunoreactivities by NR4 were localized in the submucous plexuses but not in the myenteric plexuses, and those by 2F11 were localized in both plexuses. There were fewer SY-localizations in muscle layers. In the case 2, NF-immunoreactivities by NR4 and 2F11 were very weak in the myenteric plexuses but were seen in the submucous plexuses. SY-immunoreactivities were localized in both plexuses, but not in the muscle layers. Conclusion : NF was rich in both quantity and quality in hypertrophic axons of the aganglionic bowel. These axons might innervate smooth muscle layers but the density of innervation decreased in the proximal bowel as indicated by SY localizations. In the bowel of hypoganglionosis, the structure of NF was abnormal to various degrees in the myenteric plexuses of bowels with hypogan-gionosis. Innernation in smooth muscle layers was also abnormal in the case 2.
During the past 2-year period, January 1988 to December 1989, Nissen fundoplication was performed on eleven neurologically impaired children. Preoperative symptoms and esophageal functions in these patients are discussed in this reports. All patients showed free reflux on barium esophagograms and positive acid reflux on intraesophageal 24-h pH monitoring. Manometric study revealed that basal lower esophageal sphincter (LES) tone was in inverse correlation with the age of the patients. In these patients, the antireflux function of the LES was decreased by various specific factors, i, e, kyphoscoliosis, extensor spasms and long-term supine position. Furth-ermore, an abrupt elevation of intragastric pressure associated with convulsions may become a promoting factor for a "vicious cycle" of GER in these patients. As regards indications for surgery in GER in neurologically impaired children, it is important to note that antireflux functions tend to get worse during the clinical course.
We have operated on 21 children with a buried penis using the six V-flap repair method (by Senoh) during the last 5 and a half years. This operation is easy to perform and the functional and cosmetic results are excellent, since temporary edema and narrowing of the ring of the foreskin rarely occur.
This study is a review of our patients with gastroesophageal reflux who were severely retarded. Twelve mentally retarded patients who had undergone Nissen fundoplication and pyloroplasty during a period from 1980 through 1989 are the subjects of this study. Eight patients had cerebral palsy, two had Cornelia de Lange syndrome, one had West syndrome and the other had multiple minor anomalies. Eight patients were badly undernourished and their body weights were more than 2.7 standard deviations below the mean value. Vomiting stopped in ten patients after surgery, but wheezing persisted in three of them. Weight gain was observed in eight patients. Three patients have died from unknown causes. This study suggests : (1) Severely retarded children are at high risk of developing gastroesophageal reflux. Nissen fundoplication with pyloroplasty is effective treatment. The protective anti-reflux operation should be carried out when a tube gastrostomy is to be placed in them. (2) The patients often have hypertrophy of the left lobe of the liver, kypho-scoliosis and/or a ventricular-peritoneal shunt, which make the operation awkward.(3) Careful obsevation after surgery must be carried out.
We evaluated the maxium static pressure and extent of high pressure in the anal canals of 6 males and 6 females with a mean age of 2.9 years. The sample included 11 patients with hard stool or anal membrane stenosis who were later cured by conservative therapy within 2 months, and one 6 year old boy with a chronic anal fissure later cured by operative therapy. Maximum static pressure and the length of the anal canal in children having high pressure were measured using an open-tip manometer with a continuous infusion system. Values for maximum static pressure for all 12 patients are expressed as a percentage of the values obtained from aged-matched controls reported in the literature. Values for 11 patients were observed to be 101 ±7.1%, and the value for the 6 year old boy was observed to be 125%. The length of the anal canal in children who had high pressure was 2.1±0.5cm, similar to the 2.2cm found on the normal control group. Adult patients with anal fissure have high pressure due to abnormal activity of the internal sphincter. In treatable cases in infants and children, the anal canal pressure was normal. Apparently the cause of anal fissure is trauma caused by hard stools and anal membrane stenosis.
Involvement of the liver has been thought to be rare in patients with Wilms' tumor, but it has not been rare in our patients. Liver metastases occurred in 14.4% of our patients and they occrred in 26.7% of patinents with recurrence. The most common periods for the diagnosis of liver metastases are the first few months of the illness, the period immediately after stopping chemotherapy, or any time that lung metastases are identified. Liver ultrasonography is recommended at the initial investigation every three months for the first two years, and abnormal cases should be subjected to computed tomography or angiography. Chemotherapy and radiation therapy are not very effective, and early detection and surgical treatment are needed to obtain a cure. Generally the prognosis is very poor and we have observed a cure in only one out of four patients.
In the last 20 years, 147 patients with thoracic surgical diseases in infants and children beside tracheobronchial varieties were seen at the Kobe Children's Hospital. Of these 147 patients, there were 45 patients with diseases of lung parenchyma, including CCAM. This paper summarizes our experiences in the management of pediatric lung cysts. Thirteen out of the 45 patients were clinically observed to have air cyst. CCAM was pathologically diagnosed from the beginning in only 4 but later careful review of the tissue specimen in 3, which had been interpreted as bronchogenic cyst. Review of these 7 Patients disclosed that 1) Cystic disease of the lung occurring in the early neonatal period without infectious history should be managed with the working diagnosis as CCAM. 2) Cystectomy or partial resection of the lung segment is inadequate in CCAM, leaving a risk of recurrence. 3) Segmentectomy or lobectomy which includes the lesion is recommended in the management of CCAM.
During the period from September 1978 through Decemder 1987, 81 children underwent appendectomy for acute appendicitis in our surgical section. In 47 of them (58.0%), the disease was gangrenous, mostly with panperitonitis and/or localised abscess. Nineteen (49.7%) of those suffered from vafious postoperative complications, while the other 34 children with catarrahric or phlegmonous appendicitis had no postoperative complications. Statistically, these complications occured more frequenty in patients with duration of symptoms for 5 or more days. Following next 2 years, internal appendectomy, which is a delayed surgical intervention 2-3 months after an initial conservative treatment, was tried in 6 children with a gangrenous appendicitis diagnosed at 5 or more days after the oneset of symptoms. As the results, we could improve the postoperative complication rate to 23.1% in 13 children with gangrenous appendicitis.
It is essential to clean the colon for accurate endoscopic observation. A modified version of Brown's method (conventional method) has been widely used for colonoscopy preparation in pediatric patients. We have been using a polyethylene glycol electrolyte lavage solution (GOLYTELY) since 1985. We studied the effectiveness of these two methods in 40 patients. The results were as follows : 1) The conventional method of cleansing the colon was not effective in 19% of the patients. Preparations using GOLYTELY were carried out with safety and results were more satisfactory than with the conventional method. 2) Administration of GOLYTELY was best when (1) infused from a naso-gastric tube in the prone position, (2) with an infusion speed of 15ml/kg/hour, (3) a total volume of 75ml/kg and (4) when the examination was started 5-10 hours after the completion of the infusion.
A review of aspiration of hydroceles in pediatric patients is presented. The aspiration of hydroceles fluid from a tunica vaginalis testis in communicating hydroceles is usually unsuccessful since the fluid recollects due to the patency between the peritoneum and the tunica vaginalis testis. The aspiration of hydroceles is ineffective in neonates and infants because the majority resolve spontaneously and the procedure delays spontaneous healing. The aspiration of hydroceles may only be required as a supportive treatment for non-communicating giant hydroceles in older age groups.
Tracheomalacia can induce respiratory problems and life-threatening events in postoperative patients with tracheoesophageal fistula (TEF) . Aortosternopexy was performed on six patients with tracheomalacia following repair of TEF over the past 8-year period, from December 1981 to December 1989, Clinical features include repeated respiratory infection, apparent life threatening event and failure to thrive. Tracheomalacia was confirmed by bronchoscopy (Storz) and lateral cinegraphy of the air tracheogram. The operative procedure of aortosternopexy and the results are described. The operative results were satisfactory making this procedure a treatment of choice for tracheomalacia. Further study, especially of indications, is necessary.
A case of 5-year-old boy with pancreatoblastoma was reported. He was admitted to our hospital with a complaint of abdominal distension. A massive mass was palpated just below the xiphoid process on admission. Laboratory examination revealed elevated serum AFP (561.7ng/ml). X-CT and US showed an abnormal mass occupying the whole extent of the abdomen. Open biopsy was performed even though neuroblastoma was suspected preoperatively because of negative serum NSE and VMA. The tumor microscopically showed squamoid corpuscles surrounded by an acinar-like arrangement which is referred to as an organoid structure, Pancreatoblastoma was diagnosed histologically. Distal partial pancreatectomy concomitant with splenectomy and extirpation of the tumor was performed. Cyclophosphamide and ADM were administrated immediately after the operation and radiation therapy was performed 21 days after surgery. Serum AFP decreased from 561.7ng/ml to 0ng/ml. These combined therapies were useful in treatins the pancreatoblastoma.
Leiomyomas rarely develop in the deep soft tissues except in the uterus and the gastrointestinal tract. Leiomyomas are found chiefly in adults and rarely found in children. In this paper, a child with multiple leiomyomas of the omentum and the mesentery is reported. A 6-year-old boy was admitted to our hospital with symptoms of abdominal pain and nausea following blunt trauma. Abdominal ultrasonography and CT scan revealed a solid mass in the right upper quadrant of the abdomen. An upper GI series showed two filling defects in the duodenum. Superior mesenteric arteriography revealed arterial stretching and neovascularization in the peripheral area of the gastroduodenal artery. The preoperative diagnosis was an omental neoplasm such as leiomyoma or leiomyosarcoma. At surgery, six solid tumors ranging 1 to 5 cm in diameter were located in the omentum and the mesentery. All of the tumors could be easily removed from their attachments. Microscopic exanimation confirmed the diagnosis of multiple leiomyomas. His postoperative course has been uneventful without evidence of recurrence four years after surgery.
Two surgical cases of intestinal obstruction in children due to trichophagia, a form of pica are reported with a reference to the literature. Patient I, a 2.5-year-old girl was admitted to the hospital with a chief complaint of vomiting. An upper gastrointestinal series showed duodenal dilatation with complete obstruction in the 3rd portion of the duodenum. Exploratory laparotomy revealed a soft mass in the dilated duodenum with a windsock web. This was composed of cotton or wool threads and strings. Patient II, a 5-year-old girl presented with abdominal pain and vomiting. Upright films of the abdomen showed multiple loops of small bowel with air fluid levels. She went to surgery for removal of a trichobezoar 6.0×3.0cm in the small intestine 150cm proxymal to the end of ileum. Iron deficiency anemia was not seen in either patient, and they were refered to the outpatient psychiatry clinic, and they no longer have hair pulling and trichophagia.
Bronchopulmonary foregut malformation (BFM) is an anomaly which is caused by an anomalous embryology of the foregut. We experienced a case of BFM in which the right main stem bronchus was communicating with the esophagus. This 16-day-old male infant showed tachypnea from birth. Respiratory sound was not audible in the right lung field. Esophagogram revealed a bronchus-like structure arising from the esophagus and spreading to the right hemithorax. Esophagoscopy revealed a fistula on the right posterior wall of the esophagus. Operation was performed on the 23rd day after birth. The right lung consisted of only one lobe. It communicated with not the trachea, but with the lower esophagus by a fistulous tract that seemed to be the right main stem bronchus. Blood supply flowed from the right pulmonary artery. The right lung was removed. Pathological examination revealed the presence of mature bronchioles, alveolars and vessels in the right lung tissue. The lumen of the tract communicating with the esophagus was lined with stratified columnar epithelium and the cartilage was present in the fistulous wall. Since the operation, the patient has had recurrent pneumonitis, and is diagnosed as having right pneumonectomy syndrome.
This is a case report of a 3 year-old-boy with dumbbell type neuroblastoma of III clinical stage. His first symptom was claudication of the right leg. Claudication of the left side was also observed 10 days later. First, laminectomy (T3 to T7) was undertaken to salvage the function of both legs. The tumor was resected through right thoracotomy 25 days after the laminectomy combined with intraoperative radiation therapy. Chemotherapy according to Al-protocol had also been given. Functions of both legs have been improved during 3 year and 3 months after laminectomy. We reviewed 46 cases of dumbbell type neuroblastoma reported in Japan. Having analyzed these cases, we have found following facts, 1) the incidence was 1.4 times higher in boys than in girls, 2) prognosis is better in the cases younger than 2 year-old, 3) no functional improvement could be expected without decompression laminectomy within 6 weeks after spinal symptoms.
This report concerns a case of an extensive aganglionosis having only 55cm of ganglionic jejunum below the ligament of Treitz. Five laparotomies were conducted, including the jejunostomy at 17 days of age, and the definitive operation by the Martin's method at 1 year and two months age. Intravenous hyperalimentation was applied for 3 years and 8 months, starting at 14 days of age. the patient, 7 years old at present, maintains 10% growth through oral intake alone and leads a normal social life. Based on the examination of postoperative bowel movement and capacity of absorption, the surgical managements we employed in our case can be regarded as one of the effective methods in extensive aganglionosis having ganglionic intestine shorter than 70cm from the ligament of Treitz.
Multiple duplication of the alimentary tarct is a rarecondition. This is a report of an 11-month-old boy with the multiple duplication of the colon with a perineal cutaneous fistula and multiple complicated anomalies. The patient was admitted to Kyushu University Hospital with a high fever and abdominal distension. A tubular duplication of the colon was noted preoperatively by fistulography. Two cystic duplications were also found in the transverse colon upon laparotomy. Only 20 cases with multiple duplication have been reported in Japan. Almost all of the cases developed as the duplications with the same form. The combination of different forms (cystic and tubular) as seen in this case has been previously reported in only 4 cases. There have only been 2 cases with the duplication of the alimentary tract with a cutaneous fistula. It is thought that duplication of the alimentary tract should be dissected because it can cause intestinal obstruction, hemorrhage, or malignancy.
Four cases of congenital esophageal stenosis associated with esophageal atresia and tracheoesophageal fistula (TEF) are reported. Distal esophageal stenosis was diagnosed at the time of correction of TEF in two patients, as an 8Fr tube could not pass throgh the distal esophagus. The other two patients were diagnosed in contrast studies, one at the age of 2 years and the other at the age of 8 days. Bougienage and/or pneumodilation were attempted in three patients, but the outcome was not satisfactory in all cases. Surgery was performed in four cases. Longitudinal myotomy was effective for dilatation in the patient with stenosis in the middle esophagus. The three patients with lesions near the E-G Junction needed fundoplication for maintaining the antireflux mechanism of the fundus. Myotomy with Nissen's fundoplication was performed in one patient and a resection of the lesion and esophagoesophagostomy with Collis-Nissen's fundoplication in the other two patients. The outcome of the operations was satisfactory in three cases, although the patient with Collis-Nissen's fundoplication still can not eat enough orally. Histological studies showed stenosis due to fibromuscular thickening in two cases and to tracheobronchial remnants in the other two cases. A careful investigation at/after radical TEF surgery is recommended to find stenosis.